Systemic sclerosis (SSc)

Systemic sclerosis (SSc) is a rare, autoimmune connective tissue disease characterized by widespread microvascular damage, induration and thickening of the skin and fibrotic changes in the organs, predominantly in the lungs, heart, and intestinal tract.

SSc is a heterogenous disease, ranging from stable disease for many years to very rapidly, progressive disease resulting in extensive fibrosis of skin and organs and death. Currently, SSc has a poor prognosis and cannot be cured. Very limited effective treatments are available to prevent progression of the disease. The pathobiology and heterogenous character of the disease are not well understood. As a consequence, we cannot accurately predict the disease course in the individual patient.

SSc is a heterogenous disease, ranging from stable disease for many years to very rapidly, progressive disease resulting in extensive fibrosis of skin and organs and death. Currently, SSc has a poor prognosis and cannot be cured. Very limited effective treatments are available to prevent progression of the disease. The pathobiology and heterogenous character of the disease are not well understood. As a consequence, we cannot accurately predict the disease course in the individual patient.

SSc is a main topic of research in the department of Rheumatology of the Leiden University Medical Center. The research group is headed by dr. Jeska de Vries-Bouwstra (clinical research), in close collaboration with dr. Cynthia Fehres and dr. Hans Ulrich Scherer (translational research).

As a research group we aim to increase understanding of SSc pathobiology and clinical heterogeneity, and improve health care for SSc.

To achieve this we focus on:

  1. the role of SSc-specific autoantibodies and auto-reactive B-cells in disease development and progression,
  2. biomarkers that can improve patient prognostication, with a focus on autoantibodies
  3. integrated, multidisciplinary patient-centered health care for SSc

One of the major projects of the Leiden SSc group is the establishment of the Leiden CCISS (Combined Care in Systemic Sclerosis) cohort study: an ongoing, prospective cohort study.

In our research team we apply an integrated approach with close collaboration between basic research and patient care, and collaboration between disciplines (immunologists, rheumatologists, pulmonologists, cardiologists). In addition we have established national collaboration within ARCH and international collaborations within EUSTAR, and ERN Reconnet.

Our team members

Principal investigators

  • dr. J.K. de Vries – Bouwstra
  • dr. C.M. Fehres
  • Dhr. Dr. H.U. Scherer